Superior vena caval approach for resecting intracardially extended Ewing sarcoma.

Intracardiac extension of Ewing sarcoma is extremely rare. Herein, we report the case of a pediatric patient with mediastinal Ewing sarcoma which extended to right atrium via the azygos vein. Surgical resection was performed through longitudinal incision on anterior surface of the superior vena cava under cardiopulmonary bypass. Resection was feasible because the tumor was sufficiently elastic and non-adherent to the inner surface of the heart. The patient was received chemotherapy and proton beam radiation postoperatively and is doing well with no tumor recurrence for 5 years after surgery.

Pericardial effusion after congenital heart surgery.

Objective: Pericardial effusion after cardiac surgery remains an important cause of morbidity and mortality. We describe the risk factors of pericardial effusion after congenital heart surgery through analyzing data from a nationwide, multi-institutional registry. Methods: The Japan Congenital Cardiovascular Surgery Database, which reflects routine clinical care in Japan, was used for this retrospective cohort study. Multivariable regression analysis was done after univariable comparison of patients with pericardial effusion and no pericardial effusion. Results: The study enrolled 64,777 patients registered with the Japan Congenital Cardiovascular Surgery Database between 2008 and 2016; 909 of these had postoperative pericardial effusion (1.4%) and were analyzed along with 63,868 patients without pericardial effusion. Univariable analysis found no difference between the groups in terms of gender, early delivery, or preoperative mechanical ventilatory support. In the pericardial effusion group, cardiopulmonary bypass use was lower (58.4% vs 62.1%), whereas the cardiopulmonary bypass time (176.9 vs 139.9 minutes) and aortic crossclamp time (75.1 vs 62.2 minutes) were longer, and 30-day mortality was higher (4.1% vs 2.2%). Multivariable analysis identified trisomy 21 (odds ratio, 1.54), 22q.11 deletion (odds ratio, 2.17), first-time cardiac surgery (odds ratio, 2.01), and blood transfusion (odds ratio, 1.43) as independent risk factors of postoperative pericardial effusion. In contrast, neonates, infants, ventricular septal defect, atrial septal defect, tetralogy of Fallot repair, and arterial switch operation were correlated with a low risk of pericardial effusion development. Conclusions: The incidence of postoperative pericardial effusion in congenital cardiac surgery was 1.4%. Trisomy 21, 22q.11 deletion, first-time cardiac surgery, and blood transfusion were identified as the principal factors predicting the need for pericardial effusion drainage.

Infarction induced myocardial apoptosis and ARC activation.

BACKGROUND: Apoptosis is thought to play a role in infarction induced ventricular remodeling. Apoptosis repressor with caspase recruitment domain (ARC) has been shown to limit cardiomyocytes apoptosis; however, its role in the pathogenesis of heart failure is not established. This study examines the regional and temporal relationships of apoptosis, ARC, and remodeling. METHODS: Myocardium was harvested from the infarct borderzone and remote regions of the left ventricle (LV) at 2 (n=8), 8 (n=6), and 32 (n=5) wk after MI. Activated ARC was compared with myocardial apoptosis in each region at each time. Both were then compared with the progression of remodeling. RESULTS: LV systolic volume increased by a factor 1.56±0.06 and 2.09±0.07 at 2 and 8 wk, respectively then stabilized by 32 wk (2.08±0.18). Activated ARC was elevated at 2 wk, diminished at 8 wk, and increased again at 32 wk in both regions. Apoptosis was elevated at 2 wk, and further increased at 8 wk. By 32 wk, apoptosis had diminished significantly. CONCLUSIONS: In a large animal infarction model, remodeling varied directly with the degree of apoptosis and inversely with ARC activation, suggesting that ARC acts as a natural regulatory phenomenon that limits apoptosis induced ventricular remodeling.

Sex-related resistance to myocardial ischemia-reperfusion injury is associated with high constitutive ARC expression.

The female sex has been associated with improved myocardial salvage after ischemia and reperfusion (I/R). Estrogen, specifically 17beta-estradiol, has been demonstrated to mediate this phenomenon by limiting cardiomyocyte apoptosis. We sought to quantitatively assess the effect of sex, ovarian hormone loss, and I/R on myocardial Bax, Bcl-2, and apoptosis repressor with caspase recruitment domain (ARC) expression. Male (n = 48), female (n = 26), and oophorectomized female (n = 20) rabbits underwent 30 min of regional ischemia and 3 h of reperfusion. The myocardial area at risk and infarct size were determined using a double-staining technique and planimetry. In situ oligo ligation was used to assess apoptotic cell death. Western blot analysis was used to determine proapoptotic (Bax) and antiapoptotic (Bcl-2 and ARC) protein levels in all three ischemic groups and, additionally, in three nonischemic groups. Infarct size (43.7 +/- 3.2%) and apoptotic cell death (0.51 +/- 0.10%) were significantly attenuated in females compared with males (56.4 +/- 1.6%, P < 0.01, and 4.29 +/- 0.95%, P < 0.01) and oophorectomized females (55.7 +/- 3.4%, P < 0.05, and 4.36 +/- 0.51%, P < 0.01). Females expressed significantly higher baseline ARC levels (3.62 +/- 0.29) compared with males (1.78 +/- 0.18, P < 0.01) and oophorectomized females (1.08 +/- 0.26, P < 0.01). Males expressed a significantly higher baseline Bax-to-Bcl-2 ratio (4.32 +/- 0.99) compared with females (0.65 +/- 0.13, P < 0.01) and oophorectomized females (0.42 +/- 0.10, P < 0.01). I/R significantly reduced Bax-to-Bcl-2 ratios in males. In all other groups, ARC levels and Bax-to-Bcl-2 ratios did not significantly change. These results support the conclusion that in females, endogenous estrogen limits I/R-induced cardiomyocyte apoptosis by producing a baseline antiapoptotic profile, which is associated with estrogen-dependent high constitutive myocardial ARC expression.

Adult ALCAPA: from histological picture to clinical features.

BACKGROUND: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly that results in high mortality if left untreated. Our aim was to extend our knowledge of the histological, angiographic, and clinical characteristics of ALCAPA in order to deepen our understanding of this rare entity. CASE PRESENTATION: We were involved in the assessment, treatment, and pathological evaluation of two adult ALCAPA patients who were rescued from ventricular fibrillation and then surgically treated to establish a dual coronary artery system. Histological studies indicated various chronic ischemic changes in the myocardium, patchy fibrosis, and severely thickened arteriolar walls in both ventricles. The first patient is alive and well 11.5 years after surgical correction without any implantable cardioverter defibrillator (ICD) activations. The second patient required re-do surgery 9 months after the initial operation but subsequently died. Histologically, chronic ischemic alteration of the myocardium and thickened arteriolar walls persisted even after surgical correction, and coronary angiography (CAG) showed an extremely slow flow phenomenon even after surgical correction in both patients. The average postoperative opacification rate in the first case was 7.36 + 1.12 (n = 2) in the RCA, 3.81 + 0.51 (n = 3) in the left anterior descending (LAD) artery, and 4.08 + 0.27 (n = 4) in the left circumflex (LCx) artery. The slow flow phenomenon may represent persistent high arteriolar resistance in both ventricles. CONCLUSIONS: Seldom reported or new findings in adult ALCAPA were identified in two cases. More frequent diagnosis of adult ALCAPA can be expected because of the widespread availability of resuscitation and more advanced diagnostic modalities. Accumulation of pathological and clinical findings and confirmation of the long-term follow-up results after treatment may contribute to expanding our knowledge of this rare entity and establishing optimal treatment.

A modification of extended aortic arch anastomosis augmented with subclavian flap aortoplasty for interrupted or hypoplastic aortic arch.

BACKGROUND: Surgical repair for hypoplastic aortic arch in neonates carries a substantial risk of recurrent obstruction. Simple arch anastomosis is not always a solution in cases of extended arch hypoplasia. We present our modified technique of extended aortic arch anastomosis augmented with subclavian flap aortoplasty. METHOD: We describe two neonates: interrupted aortic arch and transverse arch hypoplasia associated with aortic coarctation, who underwent a modification of extended aortic arch anastomosis augmented with subclavian flap aortoplasty. RESULTS: The patients recovered without any pressure gradient at the anastomotic site. Postoperative aortography showed no arch obstruction and they successfully underwent second stage repair. CONCLUSION: Our technique provides extensive augmentation of the aortic arch with a tension-free, wide and non-circumferential suture line which preserves potential for growth. The technique described may avoid persistent or repeat arch obstruction.

Predictors of Pericardial Effusion in Patients Undergoing Pulmonary Artery Banding.

BACKGROUND: Although pulmonary artery banding (PAB) is a common palliative procedure for pediatric heart malformation, there are concerns of pressure overload and concomitant immune reactions in the right ventricle causing postsurgical complications such as pericardial effusion. At this time, no clear guidelines as to potential risk factors or procedural contraindications have been widely disseminated. Therefore, a study was undertaken to examine wide-ranging factors to find potential biomarkers for postsurgical pericardial effusion formation risk. METHODS: A retrospective study was conducted on all cardiac surgeries performed over an eight-year period, and the main inclusion criterion was pericardial effusion development after PAB that required surgical drainage. Nine cases were then analyzed against a control group of 45 cases with respect to body measurements, concomitant surgeries, genetic screens, laboratory tests results, and cardiac function parameters. RESULTS: Trisomy 21 was strongly associated with the development of severe pericardial effusion after PAB, and postoperative serum albumin levels in patients with trisomy 21 were associated with pericardial effusion development. Other parameters showed no significant correlation with pericardial effusion development. CONCLUSIONS: Our data indicate a strong association between trisomy 21 and pericardial effusion requiring drainage after PAB, which is in line with translational research findings. Pressure overload from PAB may play a role in the formation of severe pericardial effusion that is exacerbated by cardiac structural defects commonly associated with trisomy 21. Surgical teams should therefore use caution and plan to implement drainage in PAB cases, and postoperative serum albumin may serve as a useful biomarker for pericardial effusion formation.

Patency of Saphenous Vein Grafts Using the PAS-Port System During Coronary Artery Bypass Surgery.

BACKGROUND: Several proximal anastomosis devices have been developed to shorten the time required for a proximal anastomosis and to avoid aortic cross-/side-clamping during coronary artery bypass grafting. This study retrospectively examined the patency of saphenous vein grafts (SVGs) using the PAS-Port System (Cardia Inc, Redwood City, CA). METHODS: From 2004 to 2014, 451 patients underwent coronary artery bypass graft operations requiring at least 1 proximal anastomosis using a PAS-Port device. A total of 802 PAS-Port devices were used, and 95.0% (762 of 802) were implanted successfully. Among the successfully implanted anastomoses, 76.8% (585 of 762) were evaluated using coronary angiography or multidimensional computed tomography, or both. The evaluations were performed between postoperative days 4 and 3,182 (mean, 319 ± 624 days). The early (1 to 365 days) and the midterm to long-term (more than 366 days) occlusion rates were examined. A complete postoperative clinical course was recorded for 70.7% of the patients. RESULTS: Overall, 93.8% (549 of 585) of the device-dependent SVGs were patent. The patency rates of device-dependent SVGs that were 1, 2, 3, 4, 5, 6, 7, and 8 years old were 90.1% ± 1.8%, 87.1% ± 2.3%, 86.1% ± 2.5%, 82.9% ± 3.3%, 80.6% ± 3.9%, 77.2% ± 5.0%, 77.2% ± 5.0%, and 70.2% ± 8.1%, respectively. The longest follow-up period was 3,182 days (8.7 years). The occlusion rate for device-dependent SVGs tended to decrease as the number of patients accumulated. CONCLUSIONS: The PAS-Port system provided acceptable SVG patency and clinical outcome for the early and midterm to long-term. There may be a learning curve for the use of PAS-Port device that affects the device-dependent SVG patency.

Replacement of the common atrioventricular valve with floating annuloplasty in a patient with univentricular physiology.

Significant common atrioventricular valve insufficiency is associated with increased mortality and morbidity in patients with univentricular physiology. Shortly after bidirectional cavopulmonary anastomosis and concomitant valvuloplasty, valve replacement was carried out using a floating annuloplasty technique reinforced by a Dacron strip in a patient with common atrioventricular valve insufficiency. The technique applied is described in detail.

Two adult cases of Bland-White-Garland syndrome with lethal arrhythmia due to coronary steal phenomenon during physical or mental stress.

We experienced two adult cases of anomalous origin of the left coronary artery from the pulmonary artery, so-called Bland-White-Garland (BWG) syndrome, that presented with ventricular tachycardia (VT) and ventricular fibrillation during exertion in daily life. They presented to our hospital with syncope due to VT, and recovered following application of an automated external defibrillator with cardiopulmonary resuscitation. We diagnosed BWG syndrome by multi-detector computed tomography angiography and coronary angiography. We analyzed the mechanisms of lethal arrhythmias in relation to myocardial ischemia on exertion. Coronary flow modification and implantable cardioverter defibrillator implantation were performed in order to prevent future lethal arrhythmia due to myocardial ischemia. It is important to be aware of congenital heart disease in ordinary cases. .

Molecular genetic and cytogenetic analysis of a primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma.

We performed cytogenetic and molecular cytogenetic analyses of a primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma, a rare type of primary cutaneous T-cell lymphoma. G-banded analysis at initial diagnosis and recurrence revealed complex karyotype and clonal evolution reflecting genomic instability that parallels the aggressive clinical course observed. Spectral karyotyping revealed numerous structural abnormalities. SNP array-based analysis of an initial diagnostic sample revealed numerous gains and losses of chromosomal material, including loss of short arm of the chromosome 17, to which TP53 is mapped. The molecular cytogenetics and array data of this case suggest genomic instability, particularly chromosomal instability and haploinsufficiency for TP53, the latter possibly giving rise to alteration of p14ARF-Mdm2-p53 tumor suppressor protein pathway, likely to be associated with unfavorable clinical course.

[Asplenia syndrome with total anomalous pulmonary venous connection].

Three patients of asplenia syndrome with total anomalous pulmonary venous connection (TAPVC) were reported. Case 1 with exceeding pulmonary blood flow, underwent TAPVC repair and pulmonary artery banding as a first palliation before bi-directional Glenn shunt. Case 2 did not require any surgery to control the pulmonary blood flow before the simultaneous procedure of TAPVC repair and bi-directional Glenn shunt. Case 3 with decreased pulmonary blood flow underwent a complicated course with 3 times of Blalock-Taussig shunts and the repair of TAPVC to prepare for bi-directional Glenn shunt. Simultaneous repair of TAPVC with the procedure which aimed to control the pulmonary blood flow at the first palliation surgery will simplify the control of pulmonary blood flow and prepare good condition of the lung for the Fontan operation in the future.

Xenopericardial roll graft replacement for infectious pseudoaneurysms and graft infections of the aorta.

BACKGROUND: Which graft material is the optimal graft material for the treatment of infected aortic aneurysms and aortic graft infections is still a matter of controversy. Orthotopic aortic reconstruction with intraoperatively prepared xenopericardial roll grafts without omentopexy was performed as the "initial" operation to treat aortic infection or as a "rescue" operation to treat graft infection. Mid-term outcomes were evaluated. METHODS: Between 2009 and 2013, orthotopic xenopericardial roll graft replacement was performed to treat eight patients (male/female: 6/2; mean age: 69.5 [55-80] yr). Graft material: equine/bovine pericardium: 2/6; type of operation: initial 4/rescue 4; omentopexy 0. Additional operation: esophagectomy 2. Mean follow-up period: 2.6 ± 1.6 (1.1-5.1) years. RESULTS: Replacement: ascending 3, arch 1 (reconstruction of neck vessels with small xenopericardial roll grafts), descending 3, and thoracoabdominal 1. Pathogens: MRSA 2, MSSA 1, Candida 1, E. coli 1, oral bacillus 1, and culture negative 2. Postoperative local recurrence of infection: 0. Graft-related complications: stenosis 0, calcification 0, non-infectious pseudoaneurysm of anastomosis 2 (surgical repair: 1/TEVAR 1). In-hospital mortality: 2 (MOF: initial 1/rescue 1); Survival rate exclusive of in-hospital deaths (~3 y): 100 %, but one patient died of lung cancer (3.6 yr). CONCLUSIONS: Because xenopericardial roll grafts are not composed of synthetic material, the replacement procedure is simpler and less invasive than the standard procedure. Based on the favorable results obtained, this procedure may have the possibility to serve as an option for the treatment of aortic infections and aortic graft infections not only as a "rescue" treatment but as an "initial" treatment as well.

Aortopulmonary window due to balloon angioplasty after arterial switch operation.

We report 2 cases of aortopulmonary window that developed after balloon angioplasty for pulmonary artery stenosis. Both patients had undergone arterial switch operations for complete transposition of the great arteries before the angioplasty. These aortopulmonary windows were repaired through elective operations. The clinical features, diagnosis, management, and proposed mechanisms of this complication are described.

Mortality of pulmonary artery banding in the current era: recent mortality of PA banding.

BACKGROUND: The mortality of pulmonary artery banding improved significantly in the 1980s. However, we lack information on this procedure in the current era. METHODS: The results of pulmonary artery banding in 365 patients who had operations between 1966 and 2001 were reviewed. The patients were divided into three groups: (1) group 1 patients who had operations between 1966 and 1979, (2) group 2 patients who had operations between 1980 and 1989, and (3) group 3 patients who had operations between 1990 and 2001. RESULTS: Significantly younger and smaller patients have been operated on recently (mean age: group 1, 169.0 +/- 40 days; group 2, 101.8 +/- 11 days; and group 3, 69.7 +/- 8.9 days; and mean weight: 4.6 +/- 0.1, 4.1 +/- 0.1, and 3.2 +/- 0.1 kg, respectively). A decrease was found in the number of simple cardiac anomalies, such as isolated ventricular septal defects. The early mortality in the three groups was 38.3% for group 1 (65 of 187), 13.5% for group 2 (15 of 111), and 13.8% for group 3 (12 of 87). Although the mortality did not vary significantly between groups 2 and 3, it improved over time in patients weighing less than 3 kg. Multivariate analysis of group 3 demonstrated that no isolated variable, including sex, weight, and diagnosis was a significant risk factor. CONCLUSIONS: Despite the advances in perioperative management, we found no improvement in the early mortality of pulmonary artery banding during the last decade. These results will support the preference for primary repair of intracardiac anomalies in small infants. However, this operation can now be performed with the same risk even in smaller patients. We believe that pulmonary artery banding has a role in the treatment of congenital cardiac anomalies.

Palliative open heart surgery in an infant with factor VII deficiency.

An infant with factor VII deficiency underwent palliative open heart surgery for pulmonary atresia with an intact ventricular septum. No references had been found on the management of this rare coagulation disorder in infantile cardiac surgery. We describe the peri- and postoperative management with a replacement therapy including a recombinant factor VIIa concentrate. We conclude that an appropriate replacement therapy is needed to control bleeding during open heart surgery with factor VII deficiency.

High-frequency QRS potentials as a marker of myocardial dysfunction after cardiac surgery.

BACKGROUND: High-frequency QRS potentials are sensitive to myocardial ischemia. The aim of this study was to evaluate the usefulness of high-frequency QRS potentials as a marker of myocardial dysfunction after cardiac surgery. METHODS: Seventy patients undergoing coronary artery bypass grafting or heart valve surgery were involved. High-frequency QRS potentials were measured by signal-averaged electrocardiogram, and calculated as the root-mean-square voltage of the total QRS duration (RMST). The postoperative RMST was expressed as a percentage of the preoperative RMST. The mean RMST at 1 to 2 hours after removing the aortic cross-clamp was compared with the cardiac index, inotropic agents, and aortic cross-clamping time. The occurrence of ventricular tachycardia within 24 hours and the RMST at 2 postoperative days were also evaluated. Patients were divided into quartile groups from highest to lowest at postoperative RMST (groups 1, 2, 3, and 4, respectively, from maximum to minimum). RESULTS: In postoperative states, cardiac index significantly decreased in accordance with the RMST decrease in a stepwise manner, although there were no differences in cardiac index among the four groups preoperatively. Inotropic agents and aortic cross-clamping time increased as RMST decreased. A high rate of ventricular tachycardia within 24 hours and delayed RMST recovery at 2 postoperative days were seen in group 4. The curve of sensitivity and specificity showed that severe reduction (threshold, 35%) of RMST indicated low-output syndrome. CONCLUSIONS: The severe reduction of filtered high-frequency QRS potentials was related to myocardial dysfunction. Measurement of filtered high-frequency QRS potentials could become a useful, noninvasive, real-time monitor of myocardial dysfunction after surgery.

Airway obstruction by a retropharyngeal hematoma secondary to thoracic aortic aneurysm rupture.

BACKGROUND: Retropharyngeal hematoma is a rare form of pharyngeal pathology and can present as acute airway obstruction. Among the many causes of retropharyngeal hematoma, thoracic aortic rupture is extremely rare. METHODS AND RESULTS: A 78-year-old female with airway obstruction by a retropharyngeal hematoma secondary to thoracic aortic aneurysm rupture was successfully treated by total aortic arch replacement and open stent-graft insertion. CONCLUSION: Rupture of the thoracic aorta should be considered as a rare but important cause of retropharyngeal hematoma and airway obstruction.

Appropriate route selection for extracardiac total cavopulmonary connection in apicocaval juxtaposition.

BACKGROUND: A malpositioned heart with apicocaval juxtaposition may complicate the management of patients with functional single ventricles when total cavopulmonary connection is performed. We reviewed our experience with extracardiac total cavopulmonary connection in patients with apicocaval juxtaposition with a special focus on route selection and outcomes. METHODS: Of 68 patients who underwent extracardiac total cavopulmonary connection at our hospitals, 10 patients with apicocaval juxtaposition were included in this study. The mean follow-up was 40 ± 28 months. Patient demographics were compared with data on patients without apicocaval juxtaposition. RESULTS: The age at operation was 8 ± 7 years. We carefully chose conduit routes to create satisfactory fluid dynamics. The conduit was placed between the inferior vena cava and the ipsilateral pulmonary artery in 2 patients, and the conduit crossed midline in 8 patients. The mean postoperative pulmonary artery pressure was 13 ± 2 mm Hg. The surgical and postoperative data were not significantly different when compared with the patients without apicocaval juxtaposition. There were no conduit-related early or late complications except for 1 patient who had poor ventricular function. CONCLUSIONS: Extracardiac total cavopulmonary connection in apicocaval juxtaposition can be carried out with favorable midterm outcomes. The route between the inferior vena cava and the contralateral pulmonary artery should be the primary choice when the relevant pulmonary artery is in good shape. Care must be taken in regard to critical conduit oppression by the ventricle in cases with large ventricular volume or poor ventricular function.